A clinicopathological analysis of bone and soft tissue sarcoma in children and young adults: Time to adapt with the rapidly changing landscape?

Abstract

Context: Primary bone and soft-tissue sarcomas (STSs) are rare tumors, comprising <1% of overall adult cancers. Despite its heterogeneous molecular profile, multimodality management of these tumors has led to the 5-year overall survival (OS) benefit, from approximately 50% in 1970 to the range of 75%–80% presently, in the adolescent age group. Various hospital-based registries have tried to address the scarcity of data of bone and STSs in Indian context in the last decade, but the number is not encouraging enough to gauge the pattern of care of the disease in children and young adults. Aims: To analyze the predictive and prognostic factors of clinical outcome in bone and STS in children and young adults. Settings and Design: This was a retrospective, single-institutional study from a prospectively maintained database. Subjects and Methods: We enrolled biopsy-proven patients (aged 3–35 years) of bone and STS, attended at our outpatient department during the period of January 2015 to December 2017 and traced till November 2019. Follow-up time was defined by the period from the date of registration to the date of last attendance or death. Statistical Analysis Used: Univariate log-rank analysis and unpaired t-test were used to assess the potential prognostic factors for progression-free survival and OS and further validated by multivariate Cox regression analyses. Results: Tumor size, stage at presentation, and treatment modality were the significant prognostic factors for both bone and STS. Children had better OS with 3-year OS (89.7% vs. 71.8%). Conclusions: We recommend multidisciplinary management with emphasis on early intervention in these tumors.